Abstract
BackgroundFew risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis.MethodThe CARRA legacy registry recruited pediatric rheumatology patients from 55 centers across North America from 2010 through 2014, including over 650 subjects with Juvenile Dermatomyositis. We compared the demographic characteristics, clinical disease features and treatment histories of those with and without calcinosis using univariate and multivariate logistic regression.ResultsOf the 631 patients included in the analysis, 84 (13%) had a current or prior history of calcinosis. These patients were statistically more likely to have longer durations of disease prior to diagnosis and treatment, have lipodystrophy and joint contractures, and to have received intravenous immune globulin or rituximab as treatments.ConclusionsCalcinosis is found more often in patients with prolonged active disease, severe disease, and certain clinical features such as lipodystrophy and joint contractures. When these factors are combined with other known associations and predictors, groups of at-risk patients can be more effectively identified, treated and studied to improve overall outcomes.
Highlights
As the most common inflammatory myopathy of childhood, Juvenile Dermatomyositis (JDM) affects 3.2 per million children annually in the United States [1]
Actionable risk factors previously identified include a delay to treatment and/or prolonged disease duration [5, 11, 12] and initial treatment intensity [13, 14]; prognostic factors included race [15,16,17], male sex [15], underlying cardiac disease [18, 19], presence of joint contractures [18], and presence of certain myositis-specific antibodies [20, 21]; genetic risks were identified [22, 23]
A total of 654 patients with JDM were enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) legacy registry
Summary
As the most common inflammatory myopathy of childhood, Juvenile Dermatomyositis (JDM) affects 3.2 per million children annually in the United States [1]. An enigmatic source of morbidity comes from calcinosis, the dystrophic deposition of the mineral calcium hydroxyapatite in the skin, soft tissues or muscle, There have been many studies attempting to identify risk factors or associations of calcinosis in JDM patients in order to further define the at-risk patient population. In some large cohorts, no risk factors are identified [24]. Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis
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